MCID: OSC001
MIFTS: 30

Oeis Complex

Categories: Fetal diseases, Nephrological diseases, Rare diseases

Aliases & Classifications for Oeis Complex

MalaCards integrated aliases for Oeis Complex:

Name: Oeis Complex 57 58
Omphalocele-Cloacal Exstrophy-Imperforate Anus-Spinal Defect Syndrome 58
Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects 57
Omphalocele Exstrophy Imperforate Anus 70
Cloacal Exstrophy 58

Characteristics:

Orphanet epidemiological data:

58
cloacal exstrophy
Inheritance: Multigenic/multifactorial; Age of onset: Infancy,Neonatal; Age of death: normal life expectancy;

OMIM®:

57 (Updated 05-Apr-2021)
Miscellaneous:
majority of cases are sporadic
oeis is an acronym for omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects

Inheritance:
isolated cases


HPO:

31
oeis complex:
Inheritance sporadic


Classifications:

Orphanet: 58  
Rare renal diseases
Developmental anomalies during embryogenesis


Summaries for Oeis Complex

OMIM® : 57 Carey et al. (1978) gave the name OEIS complex to a combination of defects comprising omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects. This rare complex is thought to represent the most severe end of a spectrum of birth defects, the exstrophy-epispadias sequence, which, in order of increasing severity, includes phallic separation with epispadias, pubic diastasis, exstrophy of the bladder (600057), cloacal exstrophy, and OEIS complex. Very few instances of recurrence of anomalies in this cluster have been reported. (258040) (Updated 05-Apr-2021)

MalaCards based summary : Oeis Complex, also known as omphalocele-cloacal exstrophy-imperforate anus-spinal defect syndrome, is related to cloacal exstrophy and bladder exstrophy and epispadias complex. An important gene associated with Oeis Complex is MNX1 (Motor Neuron And Pancreas Homeobox 1). Affiliated tissues include kidney, colon and uterus, and related phenotypes are talipes equinovarus and anal atresia

Wikipedia : 73 Cloacal exstrophy (EC) is a severe birth defect wherein much of the abdominal organs (the bladder and... more...

Related Diseases for Oeis Complex

Diseases related to Oeis Complex via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 106)
# Related Disease Score Top Affiliating Genes
1 cloacal exstrophy 11.6
2 bladder exstrophy and epispadias complex 11.5
3 fronto nasal malformation cloacal exstrophy 11.3
4 omphalocele 10.7
5 anus, imperforate 10.7
6 bladder exstrophy 10.5
7 short bowel syndrome 10.4
8 lipomyelomeningocele 10.4
9 spina bifida occulta 10.4
10 placenta accreta 10.4
11 neural tube defects 10.4
12 gastroschisis 10.3
13 chiari malformation 10.3
14 metabolic acidosis 10.2
15 hydronephrosis 10.2
16 teratoma 10.2
17 myelomeningocele 10.2
18 abdominal wall defect 10.2
19 thoracoabdominal syndrome 10.2
20 myelocystocele 10.2
21 vesicoureteral reflux 1 10.2
22 duodenal atresia 10.2
23 patau syndrome 10.2
24 oligohydramnios 10.2
25 squamous cell carcinoma 10.2
26 liver disease 10.2
27 compartment syndrome 10.2
28 intestinal obstruction 10.2
29 46, xy disorders of sexual development 10.2
30 chromosomal triplication 10.2
31 scoliosis 10.1
32 bladder exstrophy-epispadias-cloacal exstrophy complex 10.1
33 tethered spinal cord syndrome 10.1
34 heart septal defect 10.1
35 kidney disease 10.1
36 limb-body wall complex 10.1
37 anorectal anomalies 10.0
38 catatrichy 10.0
39 craniosynostosis 1 10.0
40 meckel diverticulum 10.0
41 split-hand/foot malformation 1 10.0
42 telecanthus 10.0
43 uterine anomalies 10.0
44 arachnoid cysts, intracranial 10.0
45 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.0
46 constricting bands, congenital 10.0
47 cryptorchidism, unilateral or bilateral 10.0
48 diastematomyelia 10.0
49 femur, unilateral bifid, with monodactylous ectrodactyly 10.0
50 microcolon 10.0

Graphical network of the top 20 diseases related to Oeis Complex:



Diseases related to Oeis Complex

Symptoms & Phenotypes for Oeis Complex

Human phenotypes related to Oeis Complex:

58 31 (show all 47)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 talipes equinovarus 58 31 Frequent (79-30%) HP:0001762
2 anal atresia 58 31 Frequent (79-30%) HP:0002023
3 hemivertebrae 58 31 Frequent (79-30%) HP:0002937
4 intestinal malrotation 58 31 Frequent (79-30%) HP:0002566
5 omphalocele 58 31 Very frequent (99-80%) HP:0001539
6 hydroureter 58 31 Occasional (29-5%) HP:0000072
7 myelomeningocele 58 31 Frequent (79-30%) HP:0002475
8 bladder exstrophy 58 31 Very frequent (99-80%) HP:0002836
9 cloacal exstrophy 58 31 Very frequent (99-80%) HP:0010475
10 hydrocephalus 31 HP:0000238
11 cryptorchidism 31 HP:0000028
12 horseshoe kidney 58 Occasional (29-5%)
13 ectopic kidney 58 Occasional (29-5%)
14 renal hypoplasia/aplasia 58 Occasional (29-5%)
15 congenital hip dislocation 31 HP:0001374
16 micropenis 31 HP:0000054
17 labial hypoplasia 31 HP:0000066
18 vesicoureteral reflux 58 Very frequent (99-80%)
19 hydronephrosis 31 HP:0000126
20 abnormality of tibia morphology 58 Frequent (79-30%)
21 hip dislocation 58 Frequent (79-30%)
22 spina bifida 58 Frequent (79-30%)
23 pelvic kidney 31 HP:0000125
24 arnold-chiari malformation 31 HP:0002308
25 hypoplasia of penis 58 Very frequent (99-80%)
26 tethered cord 31 HP:0002144
27 rectovaginal fistula 31 HP:0000143
28 anteriorly placed anus 31 HP:0001545
29 ureteropelvic junction obstruction 58 Occasional (29-5%)
30 epispadias 31 HP:0000039
31 ambiguous genitalia, male 31 HP:0000033
32 absence of the sacrum 31 HP:0010305
33 renal agenesis 31 HP:0000104
34 11 pairs of ribs 31 HP:0000878
35 abnormality of fibula morphology 58 Frequent (79-30%)
36 intestinal duplication 58 Frequent (79-30%)
37 duplicated collecting system 31 HP:0000081
38 ureterocele 58 Occasional (29-5%)
39 abnormality of the fallopian tube 58 Occasional (29-5%)
40 bifid uterus 31 HP:0000136
41 abnormality of the clitoris 58 Very frequent (99-80%)
42 absent foot 58 Frequent (79-30%)
43 ambiguous genitalia, female 31 HP:0000061
44 duplicated colon 31 HP:0005223
45 absent scrotum 31 HP:0008707
46 vesicovaginal fistula 31 HP:0001586
47 sacral segmentation defect 31 HP:0008490

Symptoms via clinical synopsis from OMIM®:

57 (Updated 05-Apr-2021)
Neurologic Central Nervous System:
hydrocephalus
tethered cord
meningomyelocele
lipomyelocystoceles
chiari malformation

Skeletal Pelvis:
congenital hip dislocation
symphysis pubis diastasis

Genitourinary Kidneys:
hydronephrosis
pelvic kidney
renal agenesis

Genitourinary External Genitalia Male:
ambiguous genitalia
epispadias
small penis
abnormal or absent scrotum

Abdomen External Features:
omphalocele

Chest Ribs Sternum Clavicles And Scapulae:
11 pairs of ribs

Genitourinary Bladder:
cloacal exstrophy
vesicovaginal fistula

Genitourinary Internal Genitalia Male:
cryptorchidism

Abdomen Gastrointestinal:
anal atresia
intestinal malrotation
rectovaginal fistula
anteriorly placed anus
duplicated colon
more
Skeletal Spine:
hemivertebrae
absent sacrum
sacral segmentation defects

Genitourinary External Genitalia Female:
ambiguous genitalia
hypoplastic labia
widely separated labioscrotal folds

Genitourinary Ureters:
hydroureter
duplicated collecting system

Genitourinary Internal Genitalia Female:
bifid uterus

Skeletal Feet:
club feet

Clinical features from OMIM®:

258040 (Updated 05-Apr-2021)

Drugs & Therapeutics for Oeis Complex

Search Clinical Trials , NIH Clinical Center for Oeis Complex

Genetic Tests for Oeis Complex

Anatomical Context for Oeis Complex

MalaCards organs/tissues related to Oeis Complex:

40
Kidney, Colon, Uterus

Publications for Oeis Complex

Articles related to Oeis Complex:

(show top 50) (show all 78)
# Title Authors PMID Year
1
Cloacal exstrophy: an epidemiologic study from the International Clearinghouse for Birth Defects Surveillance and Research. 61 57
22002951 2011
2
OEIS complex associated with chromosome 1p36 deletion: a case report and review. 57 61
20101692 2010
3
OEIS complex, VATER, and the ongoing difficulties in terminology and delineation. 57 61
11807874 2002
4
Exstrophy of the cloaca and the OEIS complex: one and the same. 57 61
11251991 2001
5
OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): a review of 14 cases. 61 57
11251992 2001
6
OEIS complex--a population study. 57 61
10797425 2000
7
OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects) in monozygotic twins. 57 61
10213043 1999
8
The OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): recurrence in sibs. 61 57
1433234 1992
9
SLC20A1 Is Involved in Urinary Tract and Urorectal Development. 57
32850778 2020
10
Genetics of Bladder-Exstrophy-Epispadias Complex (BEEC): Systematic Elucidation of Mendelian and Multifactorial Phenotypes. 57
27013921 2016
11
Exstrophy of the cloaca and exstrophy of the bladder: two different expressions of a primary developmental field defect. 57
11251990 2001
12
Monozygotic twinning and structural defects. 57
501497 1979
13
Common pathogenesis for sirenomelia, OEIS complex, limb-body wall defect, and other malformations of caudal structures. 61
33522143 2021
14
Safety of delayed surgical repair of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex in infants with significant comorbidities. 61
33231719 2021
15
Re: Complex Abdominal Wall Reconstruction Combined with Bladder Closure in OEIS Complex. 61
32960723 2020
16
First trimester cerebral appearance in the presence of closed spina bifida with myelomeningocele, part of the oeis complex. 61
33033002 2020
17
Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes. 61
31549332 2020
18
Complex abdominal wall reconstruction combined with bladder closure in OEIS complex. 61
31079865 2019
19
Prenatal ultrasound-based diagnosis of fetal OEIS complex associated with lower limb polymelia and cardiac, hepatic dysplasia: A case report. 61
31788269 2019
20
[Early diagnosis of omphalocele: Prognostic value of the herniated viscera for associated anomalies]. 61
31271893 2019
21
OEIS complex: Prevalence, clinical, and epidemiologic findings in a multicenter Mexican birth defects surveillance program. 61
31042330 2019
22
Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defect complex, multiple major reconstructive surgeries needed. 61
29416290 2018
23
A rare case of OEIS complex - newer approach to diagnosis of exstrophy bladder by color doppler and its differentiation from simple omphalocele. 61
29379239 2017
24
Discordant Anomalies with Combined Features of Pentalogy of Cantrell and OEIS Complex: A Case Report in Monochorionic Twins. 61
28617177 2017
25
A Rare Case of Genital Malformation with Omphalocele, Exstrophy of Bladder, Imperforate Anus and Spinal Defect Complex-Autopsy Findings. 61
28892921 2017
26
Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects Complex: Associated Malformations in 12 New Cases. 61
27160889 2017
27
Omphalocele, Exstrophy of Bladder, Imperforate Anus and Spinal Defect Complex with Genital Anomalies in a Late Preterm Infant. 61
30787756 2017
28
Differentiating Closed Versus Open Spinal Dysraphisms on Fetal MRI. 61
27610944 2016
29
Is 1p36 deletion associated with anterior body wall defects? 61
27144803 2016
30
Penile duplication as genital abnormality in OEIS complex: a fetal autopsy case report. 61
26513516 2016
31
Fetal and neonatal presentation of OEIS complex. 61
26520699 2015
32
Multimodality Renal Failure in a Patient with OEIS Complex. 61
26495176 2015
33
Early prenatal diagnosis of the OEIS complex with different appearance in early compared with late pregnancy before spontaneous rupture of the cloacal membrane. 61
24623061 2014
34
Re: Urological outcomes in the omphalocele exstrophy imperforate anus spinal defects (OEIS) complex: experience with 80 patients. 61
24703152 2014
35
Omphalocele, exstrophy of cloaca, imperforate anus and spinal defect (OEIS Complex) with overlapping features of body stalk anomaly (limb body wall complex). 61
25400352 2014
36
Urological outcomes in the omphalocele exstrophy imperforate anus spinal defects (OEIS) complex: experience with 80 patients. 61
22640865 2013
37
OEIS complex. 61
24839537 2013
38
Prenatal findings of omphalocele-exstrophy of the bladder-imperforate anus-spinal defects (OEIS) complex. 61
22925220 2012
39
Deciphering caudal embryonic defects: embryological analysis and reviewing literature data. 61
23303026 2012
40
Prenatal diagnosis of OEIS (omphalocele, bladder exstrophy, imperforate anus, clubfeet) variant associated with increased nuchal translucency and OEIS complex with ambiguous genitalia associated with corrected transposition of the great arteries: case series and review of the literature. 61
21475965 2011
41
Gastrointestinal reconstruction and outcomes for patients with the OEIS complex. 61
21453858 2011
42
Comprehensive genetic analysis of OEIS complex reveals no evidence for a recurrent microdeletion or duplication. 61
21204209 2011
43
Prune belly syndrome associated with cloacal anomaly, patent urachal remnant, and omphalocele in a female infant. 61
21034928 2010
44
Neuro-orthopedic manifestations of the omphalocele exstrophy imperforate anus spinal defects complex. 61
20728185 2010
45
Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex in a child with nail-patella syndrome. 61
20880310 2010
46
Prenatal diagnosis and postnatal outcome of fetal spinal defects without Arnold-Chiari II malformation. 61
19688779 2009
47
Double lumbosacral lipomas of the dorsal and filar types associated with OEIS complex: case report. 61
19855150 2009
48
Supernumerary kidney in a child with OEIS complex. 61
19371928 2009
49
First-trimester prenatal sonographic diagnosis of omphalocele-exstrophy-imperforate anus-spinal defects complex. 61
18726969 2009
50
Discordant omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex and cardiac malformations in monochorionic twins. 61
19842883 2009

Variations for Oeis Complex

Expression for Oeis Complex

Search GEO for disease gene expression data for Oeis Complex.

Pathways for Oeis Complex

GO Terms for Oeis Complex

Sources for Oeis Complex

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
Content
Loading form....